Cholangiocarcinoma is an extremely rare and highly aggressive primary malignancy of the biliary tract. The current report illustrates a rare case of distal common bile duct adenocarcinoma encountered in a 65-year-old male patient who was thoroughly investigated after presenting with a two weeks history of pain in the right hypochondrium, jaundice and unintentional weight loss (6 kg in two weeks). The medical team opted for a pancreaticoduodenectomy (Whipple procedure) managing to obtain negative resection margins of the tumor with a favorable immediate postoperative evolution. However, the surgical team was forced to reintervene twice due to complications caused mainly by the patient's disregard.